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Fludarabine-Based Reduced-Intensity Conditioning Regimen for Hematopoietic Stem Cell Transplantation in Pediatric Patient With Il10 Receptor Deficiency Publisher Pubmed



Kheder M1, 2 ; Behfar M1 ; Farajifard H1, 3 ; Ali T1, 2 ; Hkima Abou Fakher F1, 2 ; Hamidieh AA1
Authors
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Authors Affiliations
  1. 1. Pediatric Cell Therapy Research Center, Tehran University of Medical Sciences, Tehran, Iran
  2. 2. Pediatric Stem Cell Transplant Department, Children’s Hospital, Damascus University, Damascus, Syrian Arab Republic
  3. 3. Immunology-Microbiology Department, Qom University of Medical Sciences and Health Services, Qom, Iran

Source: Pediatric Transplantation Published:2021


Abstract

IL-10R deficiency results in severe immune dysregulation. Herein, we describe the successful treatment of a girl aged 6.8 years with IL10R deficiency by using RIC prior to HSCT from a matched unrelated donor. The regimen was well tolerated, the engraftment was completely attained. On a follow-up of 7 months, the patient remained in good medical conditions with full donor chimerism. All complications before HSCT were completely resolved and her growth was accelerated. RIC regimen might be adequate to induce permanent engraftment and avoid severe organ toxicity in IL-10R deficiency patients. © 2020 Wiley Periodicals LLC
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